Central Precocious Puberty as a Sign of Congenital Adrenal Hyperplasia: Case Presentations

R.L Markosyan,L.V Navasardyan

doi:10.6000/1929-4247.2020.09.04.5

Abstract

Central precocious puberty results from the premature activation of the hypothalamic-pituitary-gonadal axis. Rarely congenital adrenal hyperplasia and/or its inappropriate treatment can be a peripheral cause of CPP. There are very few case reports of this etiology. Chronic mildly to moderately elevated adrenal androgens or intermittent hyperandrogenemia in congenital adrenal hyperplasia may trigger the precocious activation of the hypothalamic-pituitary axis, leading to CPP. In the current work, we describe 6 cases of late diagnosis of congenital adrenal hyperplasia associated with central precocious puberty. Central precocious puberty seems to be a complication of congenital adrenal hyperplasia, particularly in countries where a routine neonatal screening program for this condition is lacking. It is unclear whether these patients could avoid central precocious puberty development if the congenital adrenal hyperplasia was diagnosed in the neonatal period and appropriately treated. The current work underlines the need for congenital adrenal hyperplasia neonatal screening implementation and further investigation of the association of these two endocrine disorders

Highlights

Central precocious puberty (CPP) results from the early activation of the hypothalamic-pituitary-axis (HPA)
A 4 years old boy was referred to the pediatric endocrinologist with complaints of growth acceleration and pubic hair growth
At the age of 4 years, growth, bone age acceleration, and pubic hair growth were revealed, and CPP was diagnosed based on the GnRH stimulation test results

Summary

Introduction

Central precocious puberty (CPP) results from the early activation of the hypothalamic-pituitary-axis (HPA). Congenital adrenal hyperplasia (CAH) can be a peripheral cause of CPP. *Address correspondence to this author at the Yerevan State Medical University, Yerevan, Armenia; E-mail: renatamarkosyan@mail.ru records, including age, sex, clinical data, and relevant biochemical, hormonal, and radiological investigations. The diagnosis of CAH and CPP was established according to the standard criteria based on clinical, laboratory, and endocrinological evaluation and performed stimulation tests [10].

Results

Conclusion